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Drug Nexviazyme® (avalglucosidase alfa-ngpt) [Genzyme Corporation.]

April 2022

Therapeutic area - Pompe Disease

Initial approval criteria

  • Patient age is 1 year or older AND

Universal Criteria 

  • Will not be used in combination with other enzyme replacement therapies (i.e., alglucosidase-alfa) AND
  • Patient has not experienced a severe hypersensitivity reaction including anaphylaxis to alglucosidase alfa (Note: exception to this criterion can be made when Nexviazyme is used as part of a desensitization procedure) AND
  • Patient is not susceptible to fluid volume overload, or has an acute underlying respiratory illness or compromised cardiac or respiratory function for whom fluid restriction is indicated AND

Pompe disease (Acid alpha-glucosidase (GAA) deficiency)

  • Diagnosis has been confirmed by one of the following:
    • Deficiency of acid alpha-glucosidase (GAA) enzyme activity OR
    • Detection of biallelic pathogenic variants in the GAA gene by molecular genetic testing AND
  • Patient has a diagnosis of late-onset (non-infantile) disease

Renewal criteria

  • Patient continues to meet indication-specific relevant criteria such as concomitant therapy requirements (not including prerequisite therapy), performance status, etc. identified in the initial approval criteria AND
  • Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include: anaphylaxis and severe hypersensitivity reactions, severe infusion-associated reactions, acute cardiorespiratory failure, etc. AND
  • Patient is being monitored for antibody formation (including neutralizing antibodies)

Quantity limits

  • Patient weighing ≥30 kg: 20 mg/kg (of actual body weight) every two weeks
  • Patient weighing <30kg: 40 mg/kg (of actual body weight) every two weeks
  • Patient’s body weight (in kg) must be submitted at time of request

Billing for Nexviazyme

  • Nexviazyme must be billed as a medical claim


MHCP Provider Resource Center 651-431-2700 or 800-366-5411

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